Vanishing Bone Disease or Phantom Bone Disease/Gorham Stout Disease/Progressive Massive Osteolysis is a skeletal disease, which is characterized by destructive or uncontrolled proliferation of vascular/lymphatic channels within a bone and also its surrounding soft tissue.
It can also spread to contiguous bones with destructive or progressive resorption of the bone (whole or part of it). It is mostly confined to one bone in the body, but it could also be present in multiple bones.
Why Are These Lymph Vessels So Important?
The Lymphatic System is a network of channels called the lymph vessels which transport the lymph to the bloodstream. Lymph is a fluid that contains lymphocytes, fats and proteins.
It is also called the "infection-fighting fluid." This Lymphatic System also includes the spleen, which produces the bone marrow, the soft tissue inside the bone that produces blood cells.
The spleen also removes the worn out Red Blood Cells from the body and the bone marrow replenishes them. In the Vanishing Bone Disease, these lymph vessels get replaced by the fibrous connective tissue, disrupting its function. Thus, a damage to the development to this system is fatal to a living being.
What Are Its Causes?
The cause of Vanishing Bone Disease is actually unknown. Bone mass is maintained by a process, in which old bone gets dissolved and the new bone replaces it. Osteoclasts are cells that secrete certain enzymes that dissolve the old bone and Osteoblasts produce the new bone. In a person with the Vanishing Bone Disease, this process is disrupted, causing an imbalance in the bone mass.
This type of an imbalance causes some chemical changes in the body that affect the Osteoblasts. Thus, the bone that is getting dissolved is more than the bone getting formed. No immunological, genetic or environmental factors have been known to cause this disease.
What Happens To A Person With Vanishing Bone Disease?
The most commonly affected bones are - shoulder, pelvis, jaw, ribs, spine, skull and collarbone. It can be characterized by pain, external swelling and deformity of the area surrounding the bone.
But there are times when external symptoms are not seen until the bone is propelled by an external activity like a fall or extensively strenuous exercise, basically because of some form of minor trauma.
Bones involved also show a gradual reduction in the mass. This makes the bone weaker overtime, making it more vulnerable to severe bone damage.
When the spine or skull is involved, it causes neurological complications. It could lead to paralysis in a few cases. Meningitis or inflammation of the meninges is also a possibility.
When the ribs are involved, Chylothorax or accumulation of Chyle in between the membranes that line the lungs and chest cavity occurs. Chyle is a thick fluid that is nothing but fat and lymph. This accumulation causes difficulty in breathing and chest pain.
This could become severe if any fluid leaks to the heart. Pericardial effusion or the accumulation of fluid around the heart, the pericardium particularly, could be life-threatening.
There are chances of the disease getting eliminated after a few years. But if the spine, skull or chest is involved, it proves to be quite fatal. The severity of other symptoms depends from person to person, and on which bone is involved and how extensive the damage is.
How Can It Be Treated?
Since the etiology of this disease remains undiscovered, so does a specific treatment. Though many therapeutic methods have been used on patients, there's no one method that has worked for all. The treatment for this disease is specific for each patient based on each symptom.
Till date, three major treatments have been worked with - Radiation, Surgery and Medicine Therapy.
Radiation and Surgery have been used on patients with larger lesions that have disabled the normal functioning of the bones in the body. Radiation has seen to reduce pain and restrict further spreading of this disease.
It is also a proven treatment for Chylothorax. But it can only be used as a temporary, moderate-dose method, because it was seen to cause other complications, like growth restriction and secondary malignancy.
Surgery involves resection and reconstruction of the lesion by either removing the affected part or by using prostheses or bone grafts to initiate bone growth.
Medical Therapy involves the use of biphosphonates, which show antiosteolytic activity. Other agents like a-2b interferon, androgens, calcium, adrenal agents, cisplatin, oestrogen, magnesium, bleomycin, fluoride, calcium and vitamin D have also been suggested, but with inconsistent results.
Research is being carried out to target pathways of receptors of lymphangiogenic growth factors to treat this disease more effectively.
Though the Vanishing Bone Disease is a rare occurrence, the limited knowledge in its treatment is what makes it scarier.
"Awareness is the greatest agent for change."
Be aware, it is crucial for your body.
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